Clinical correlates to the goniodysgensis among juvenile-onset primary open-angle glaucoma patients

To evaluate gonioscopic features and relate them to clinical characteristics in eyes with juvenile-onset primary open-angle glaucoma (JOAG). Goniophotographs of unrelated JOAG patients, presenting between 10-40 years of age, were evaluated and compared with 60 healthy subjects in the same age group. Age of onset, family history of glaucoma, highest untreated IOP and visual field defect (mean deviation) were analyzed and correlated with the gonioscopic features among JOAG patients. Of 126 patients included in the study, 44 (34 %) had a normal open angle (group 1), while 82 (66 %) had developmental anomalies (group 2). Developmental anomalies of the angle were classified as: high iris insertion with or without prominent iris processes (n = 42), a featureless angle (n = 30), and those with prominent iris processes alone (n = 10). There was no difference in age of onset (group 1, 30.5 +/- 7 years and group 2, 26.3 +/- 9.6 years) (p = 0.07) or the untreated IOP at presentation (group 1; 36 +/- 12.5 mmHg and group 2, 38.8 +/- 12.3 mmHg; p = 0.37) between the groups. However, those with angle anomalies presented with a greater visual field defect (MD -23.5 +/- 10.5 vs -14.8 +/- 13 dB; p = 0.02) compared to those with normal appearing angle. While two thirds of JOAG patients present with developmental anomalies of the angle, one third have normal appearing angles. High insertion of the iris is the most common form of gonio dysgenesis observed. Those with angle dysgenesis are more likely to present with severe disease.